[Human chorionic gonadotropin-secreting gonadoblastomas in a girl of 45, X Turner syndrome: a case report and literature review].

Objective: To summarize the experience in diagnosis and treatment of 45, X Turner syndrome (TS) with gonadal Y chromosome mosaicism and bilateral gonadoblastoma (Gb) secreting human chorionic gonadotrophin(HCG). Methods: A female patient aged 5 years and 3 months was admitted to the hospital with a complaint of "enlarged breasts for 27 months, and elevated blood ß-HCG for 8 months". The clinical data were summarized, and related literature up to March 2022 with the key words"Turner syndrome" "Gonadoblastoma" "Y chromosome" "human chorionic gonadotropin" "precocious" in PubMed, CNKI and Wanfang databases were reviewed. Results: The girl went to the local hospital for 2-month breast development at age of 3 years, and was found with a heart murmur diagnosed with "pulmonary venous malformation and atrial septal defect (secondary foramen type)". Surgical correction was performed. She experienced the progressive breast development, rapid linear growth and markedly advanced skeletal age, which cannot be explained by partial activation in the hypothalamic-pituitary-gonadal axis determined at the age of 3 years and 7 months in local hospital. Then whole-exome sequencing revealed chromosome number abnormality 45, X, which was confirmed by Karyotyping. At the age of 4 years and 6 months, serum ß-HCG was found to be elevated (24.9 U/L) with no lesion found at the local hospital. On physical examination, she was found with breast development, pubic hair development and clitoromegaly with elevated serum testosterone (1.96 µg/L) and ß-HCG (32.3 U/L). Sex determining region Y(SRY) gene was negative in peripheral blood sample. Thoracic and abdominal CT, head and pelvic magnetic resonance imaging were normal. Exploratory laparotomy confirmed the presence of a left adnexal tumor and a right fibrous streak gonad. During surgery, simultaneous samples of bilateral gonadal and peripheral venous blood were obtained and serum ß-HCG, estradiol and testosteron concentrations was higher to lower from left gonadal venous blood, right gonadal venous blood, to peripheral venous blood. Bilateral gonadectomy was performed. Histopathology revealed bilateral gonadoblastomas. SRY was positive in bilateral gonadal tissues. After surgery, serum E2, testerone and ß-HCG returned to normal. So far 4 cases of HCG-secreting gonadoblastoma had been reported worldwide. The phenotypes of the 4 cases were all female, with virilization or amenorrhea, and the preoperative peripheral blood ß-HCG concentrations were 74.4, 5.0, 40 456.0, and 42.4 U/L, respectively. Conclusions: There is a high risk of Gb in TS with Y chromosome components. Gb is infrequently presented with breast development, and Gb associated with HCG secretion is rare. Karyotyping should be performed in a phenotypic female with masculinization, and virilization in TS indicates the presence of Y chromosome material with concurrent androgen secreting tumors.

Formation and verification of the structure of the 1-fluorenylmethyl chloroformate derivative of sulfamethazine.

Sulfamethazine (SMZ) is derivatized with 1-fluorenylmethyl chloroformate (FMOC) to form the fluorescent adduct SMZ-FMOC. Conditions for formation are optimized with respect to pH, reagent concentration, and reagent ratio. Reagent and product profiles (including the hydrolysis byproduct FMOC-OH) versus time are followed by reversed phase HPLC with UV absorbance detection. FMOC-SMZ has been crystallized, its composition confirmed by microanalysis, and its structure corroborated by IR and NMR spectroscopy. From 10 down to 1 ppm, there is clear gentle curvature in the fluorescence intensity of SMZ-FMOC. The linear response range extends from above 100 ppb down to about 100 ppt, and an increase in sensitivity for the fluorescent detection of FMOC-SMZ (over the usual UV absorbance detection of SMZ) is calculated to be better than 3 orders of magnitude.

An evaluation of oral ulcers in patients with AIDS and AIDS-related complex.

BACKGROUND: Patients with HIV infection can have recurrent and persistent oral ulcers, not attributable to known infectious agents. OBJECTIVE: Our aim was to evaluate prospectively oral ulcers in patients with HIV infection to determine whether an etiologic agent could be identified. METHODS: Sixteen patients with HIV infection who had oral ulcers not attributable to known causes had culture of the base and a biopsy specimen taken from the ulcer. Cultures were obtained for herpes simplex and varicella-zoster viruses, mycobacteria, and fungi. By polymerase chain reaction (PCR) analysis with primer/probe sets for herpes simplex viruses 1 and 2, varicella-zoster virus, cytomegalovirus, human papillomavirus, and Mycobacterium tuberculosis, each biopsy specimen was analyzed for the presence of DNA from these organisms. Specimens were also evaluated histologically. RESULTS: Histoplasmosis was detected histologically in one biopsy specimen, candidiasis in a second, and herpetic changes in a third. Viral cultures were positive for herpes simplex virus 1 in four cases and herpes simplex virus 2 in one case. PCR analysis detected DNA for herpes simplex virus 1 in one case and herpes simplex virus 2 in another; DNA from other pathogens was not identified. In the remaining eight patients, hematoxylin-and-eosin staining revealed eosinophilic ulcers in five cases and nonspecific changes in three cases. CONCLUSION: The etiologic agent of recurrent or persistent oral ulcers in patients with AIDS and AIDS-related complex was not identified in 50% of patients. PCR analysis was not useful. Herpes simplex virus or other pathogens were not detected in ulcers containing numerous eosinophils.